Storming the castle: A case report of multi-system dysregulation in a child with Castleman disease.

Castleman disease is a non-clonal, lymphoproliferative disorder rarely seen in children. Presented is a 12-year-old male with progressive abdominal pain, vomiting, and fever. Diagnostic testing revealed multi-organ system involvement and the diagnosis was ultimately made with tissue biopsy. Marked disease regression occurred after high-dose steroids and continued interleukin-6 inhibition.

Initial resuscitation of a neonate with congenital maxillomandibular syngnathia: A case report and literature review.

We report a full-term male neonate found to have undiagnosed syngnathia requiring extensive resuscitation at birth followed by urgent tracheostomy. We conducted a systematic literature review to study the presentation, resuscitation methods, and outcomes of neonates with congenital syngnathia. Of the 174 cases reported to date, 91 had initial resuscitation data available. Extensive resuscitation was required in 16 of these 91 infants (18%). This ranged from nasal intubation to emergent tracheostomy. These neonates are potentially higher risk deliveries for which methods in addition to those recommended by the American Heart Association neonatal resuscitation guidelines may be needed.

Pediatric Osteopathic Manipulative Medicine: A Scoping Review.

CONTEXT: A common reproach precluding the use of osteopathic manipulative medicine (OMM) in pediatrics is a lack of evidence regarding its safety, feasibility, and effectiveness. OBJECTIVE: We conducted a systematic, scoping review of pediatric osteopathic medicine to identify gaps in the literature and make recommendations for future research. DATA SOURCES: We searched 10 databases using 6 key words and medical subject heading terms for any primary articles reporting OMM use in children published from database inception until initiation of the study. STUDY SELECTION: Articles were selected if they reported primary data on OMM conducted in the United States on patient(s) 0 to 18 years old. DATA EXTRACTION: Baseline study characteristics were collected from each article and the Grading of Recommendations, Assessment, Development, and Evaluations system was used to critically appraise each study. RESULTS: Database search yielded 315 unique articles with 30 studies fulfilling inclusion and exclusion criteria. Of these, 13 reported the data required to demonstrate statistically significant results, and no significant adverse events were reported. The majority of studies were graded as providing weak clinical evidence because of significant methodologic flaws and biases. LIMITATIONS: The review was limited to US-based studies and reports. Minimal discrepancies between reviewers were resolved via an objective third reviewer. CONCLUSIONS: There is little strong, scientific, evidence-based literature demonstrating the therapeutic benefit of OMM for pediatric care. No strong clinical recommendations can be made, but it can be medically tolerated given its low risk profile. High-quality, scientifically rigorous OMM research is required to evaluate safety, feasibility, and efficacy in pediatrics.

Central Venous Line Associated Deep Vein Thrombosis in Hospitalized Children.

An increase in the incidence of deep vein thrombosis (DVT) has been reported in pediatric patients over the past decade. The presence of central venous line (CVL) is a major contributing risk factor with conflicting data on the relative risk of DVT with various types of central lines. We aimed to assess the incidence of and identify potential risk factors for DVT overall and with different types of CVL individually. A retrospective chart review of pediatric patients with a CVL placed at Cleveland Clinic Children's from 2011 to 2016 was conducted. Data collected included demographics, potential risk factors, CVL characteristics and related thrombotic events. The study cohort consisted of 376 CVLs in 325 patients between 0 and 26 years of age. There were 1.6 thrombi per 10,000 line-days (95% confidence interval: 1.0, 2.5), and the overall incidence of DVT was 5.1%. The incidence of DVT was highest with tunneled catheters (5/16=31%) versus with peripherally inserted central catheters (4/111=3.6%) or with ports (10/249=4%, P<0.001), and whereas there were overarching significant risk factors for CVL-associated thrombi, these risk factors differed in significance when analyzed by the CVL type. The study supports the need for continued improvement in pediatric hospital practices for early identification of patients at a higher thrombosis risk.

Impact of Race and Ethnicity on End-of-Life Experiences for Children With Cancer.

BACKGROUND: Racial and ethnic disparities in the provision of end-of-life care are well described in the adult oncology literature. However, the impact of racial and ethnic disparities at end of life in the context of pediatric oncology remains poorly understood. OBJECTIVE: To investigate associations between end-of-life experiences and race/ethnicity for pediatric patients with cancer. METHODS: A retrospective cohort study was conducted on 321 children with cancer enrolled on a palliative care service at an urban pediatric cancer who died between 2011 and 2015. RESULTS: Compared to white patients, black patients were more likely to receive cardiopulmonary resuscitation (CPR; odds ratio [OR]: 4.109, confidence interval [CI]: 1.432-11.790, P = .009) and underwent 3.136 (CI: 1.433-6.869, P = .004) CPR events for every 1 white patient CPR event. The remainder of variables related to treatment and end-of-life care were not significantly correlated with race. Hispanic patients were less likely to receive cancer-directed therapy within 28 days prior to death (OR: 0.493, CI: 0.247-0.982, P = .044) as compared to non-Hispanic patients, yet they were more likely to report a goal of cure over comfort as compared to non-Hispanic patients (OR: 3.094, CI: 1.043-9.174, P = .042). The remainder of variables were not found to be significantly correlated with ethnicity. CONCLUSIONS: Race and ethnicity influenced select end-of-life variables for pediatric palliative oncology patients treated at a large urban pediatric cancer center. Further multicenter investigation is needed to ascertain the impact of racial/ethnic disparities on end-of-life experiences of children with cancer.

Predictors of Location of Death for Children with Cancer Enrolled on a Palliative Care Service.

BACKGROUND: In the U.S., more children die from cancer than from any other disease, and more than one third die in the hospital setting. These data have been replicated even in subpopulations of children with cancer enrolled on a palliative care service. Children with cancer who die in high-acuity inpatient settings often experience suffering at the end of life, with increased psychosocial morbidities seen in their bereaved parents. Strategies to preemptively identify children with cancer who are more likely to die in high-acuity inpatient settings have not been explored. MATERIALS AND METHODS: A standardized tool was used to gather demographic, disease, treatment, and end-of-life variables for 321 pediatric palliative oncology (PPO) patients treated at an academic pediatric cancer center who died between 2011 and 2015. Multinomial logistic regression was used to predict patient subgroups at increased risk for pediatric intensive care unit (PICU) death. RESULTS: Higher odds of dying in the PICU were found in patients with Hispanic ethnicity (odds ratio [OR], 4.02; p = .002), hematologic malignancy (OR, 7.42; p < .0001), history of hematopoietic stem cell transplant (OR, 4.52; p < .0001), total number of PICU hospitalizations (OR, 1.98; p < .0001), receipt of cancer-directed therapy during the last month of life (OR, 2.96; p = .002), and palliative care involvement occurring less than 30 days before death (OR, 4.7; p < .0001). Conversely, lower odds of dying in the PICU were found in patients with hospice involvement (OR, 0.02; p < .0001) and documentation of advance directives at the time of death (OR, 0.37; p = .033). CONCLUSION: Certain variables may predict PICU death for PPO patients, including delayed palliative care involvement. Preemptive identification of patients at risk for PICU death affords opportunities to study the effects of earlier palliative care integration and increased discussions around preferred location of death on end-of-life outcomes for children with cancer and their families. IMPLICATIONS FOR PRACTICE: Children with cancer who die in high-acuity inpatient settings often experience a high burden of intensive therapy at the end of life. Strategies to identify patients at higher risk of dying in the pediatric intensive care unit (PICU) have not been explored previously. This study finds that certain variables may predict PICU death for pediatric palliative oncology patients, including delayed palliative care involvement. Preemptive identification of patients at risk for PICU death affords opportunities to study the effects of earlier palliative care integration and increased discussions around preferred location of death on end-of-life outcomes for children with cancer and their families.

Predictors of Late Palliative Care Referral in Children With Cancer.

CONTEXT: Early integration of palliative care (PC) in the management of children with high-risk cancer is widely endorsed by patients, families, clinicians, and national organizations. However, optimal timing for PC consultation is not standardized, and variables that influence timing of PC integration for children with cancer remain unknown. OBJECTIVES: To investigate associations between demographic, disease, treatment, and end-of-life attributes and timing of PC consultation for children with high-risk cancer enrolled on a PC service. METHODS: A comprehensive standardized tool was used to abstract data from the medical records of 321 patients treated at a large academic pediatric cancer center, who died between 2011 and 2015. RESULTS: Gender, race, ethnicity, enrollment on a Phase I protocol, number of high-acuity hospitalizations, and receipt of cardiopulmonary resuscitation were not associated with timing of PC involvement. Patients with hematologic malignancy, those who received cancer-directed therapy during the last month of life, and those with advance directives documented one week or less before death had higher odds of late PC referral (malignancy: odds ratio [OR] 3.24, P = 0.001; therapy: OR 4.65, P < 0.001; directive: OR 4.81, P < 0.0001). Patients who received hospice services had lower odds of late PC referral <30 days before death (OR 0.31, P < 0.001). CONCLUSION: Hematologic malignancy, cancer-directed therapy at the end of life, and delayed documentation of advance directives are associated with late PC involvement in children who died of cancer. Identification of these variables affords opportunities to study targeted interventions to enhance access to earlier PC resources and services for children with high-risk cancer and their families.

Illness and end-of-life experiences of children with cancer who receive palliative care.

BACKGROUND: The field of pediatric palliative oncology is newly emerging. Little is known about the characteristics and illness experiences of children with cancer who receive palliative care (PC). METHODS: A retrospective cohort study of 321 pediatric oncology patients enrolled in PC who died between 2011 and 2015 was conducted at a large academic pediatric cancer center using a comprehensive standardized data extraction tool. RESULTS: The majority of pediatric palliative oncology patients received experimental therapy (79.4%), with 40.5% enrolled on a phase I trial. Approximately one-third received cancer-directed therapy during the last month of life (35.5%). More than half had at least one intensive care unit hospitalization (51.4%), with this subset demonstrating considerable exposure to mechanical ventilation (44.8%), invasive procedures (20%), and cardiopulmonary resuscitation (12.1%). Of the 122 patients who died in the hospital, 44.3% died in the intensive care unit. Patients with late PC involvement occurring less than 30 days before death had higher odds of dying in the intensive care unit over the home/hospice setting compared to those with earlier PC involvement (OR: 4.7, 95% CI: 2.47-8.97, P < 0.0001). CONCLUSIONS: Children with cancer who receive PC experience a high burden of intensive treatments and often die in inpatient intensive care settings. Delayed PC involvement is associated with increased odds of dying in the intensive care unit. Prospective investigation of early PC involvement in children with high-risk cancer is needed to better understand potential impacts on cost-effectiveness, quality of life, and delivery of goal concordant care.

Knowledge about various contraceptive methods in young women with and without eating disorders.

OBJECTIVE: To determine if young women with eating disorders (EDs) have altered views about the risks/benefits of different forms of birth control than the general population. METHOD: Data was collected using a cross-sectional, survey-based study of postmenarchal women aged 13-25 years with a diagnosed ED (n = 50) or no history of disordered eating patterns (n =57). RESULTS: Despite having a higher level of education (p = 0.04) and no differences in sexual history (p = 0.16), ED patients were less knowledgeable than controls about the health risks and benefits, effectiveness in preventing HIV, and effectiveness in preventing pregnancy of various methods of birth control (p≤ 0.05). DISCUSSION: ED patients may be incorrectly presumed to be asexual while working on recovery; physicians may need to take extra time to educate ED patients about their personal risks of unintended pregnancy, sexually transmitted infections, and the benefits that different methods of contraception can provide.